New to pure red cell aplasia

[k8samp]

Hello all,
I'm Kate, 33 yo RN, mother of 3, who has just been dx "tentatively" w/PRCA. My story started about a month ago when I started experiencing a lot of tachycardia and weakness at work. It eventually reached the point where, in the middle of my shift I had to leave and go to the ER, thinking I had the flu. Upon arrival my hgb was 3.9 & my hct was 10. Shocking to say the least. Spent 3 days in hosp. got 3 units, had a *failed* bone marrow biopsy, and was sent home w/a hgb of 5.8 & a follow up in 2 days. At that appt hgb was up to 7.3 (yay), all manner of tests still pending, so once again went home w/follow up in 6 days. At that appt I went in confident, only to find I'd dropped to 5.8 again. Admitted for 2 days, 2 more units, another bone marrow biopsy- this one successful. TONS of testing for strange diseases, and discharged pending results. Now it's 3 days later, results aren't back yet but hgb this morning was up to 7.9, was put on 100 mg of prednisone daily as well, 3 days ago. I guess my question is- should I still feel so exhausted?? I've tried to research PRCA but there's not much out there.... I'm very new to this and truthfully rather scared so ANY input or advice would be truly appreciated. Thank

[Neil Cuadra]

Hi Kate.

Bone marrow failure diseases are rare and PRCA is rare among bone marrow failure diseases, which is why you don't find a lot of information about it. I think that your immediate goals should be to get a confirmed diagnosis, see if you can find a doctor who has treated PRCA before (perhaps in Rochester, Buffalo, or New York City), and then to work with the doctor(s) to find a way to keep your red blood cell count up. PRCA may often be less critically life-threatening than other bone marrow failure diseases, but the lack of patients, doctors, and research is a disadvantage.

You're lucky to live in a state with many choices of excellent treatment centers. You might consider traveling further if you can get an evaluation and recommendation from a specialist with experience.

It's no surprise that you're exhausted. Our brains and bodies are powered by our hemoglobin. When your count is low, your energy is low. Transfusions solve the immediate problem but if you need transfusions often that's not a great long-term solution and can lead to iron overload that must also be treated.

I suggest that you read this forum thread and this forum thread and this forum thread to get an idea how we use these forums to share information, and you're free to search or browse Marrowforums looking for more.

Watch this thread and you're sure to hear from triumphe64, our most active PRCA forum member, but not the only one.

The Aplastic Anemia & MDS International Foundation does its best to help PRCA patients and I suggest that you call them and ask what information they can provide to you. There's been some research on PRCA treatment (example: a study of Zenapax (daclizumab) but you need an expert to tell you what the latest research tells us and to evaluate your specific case. The AA&MDSIF may be able to suggest how best you can learn more. Medical language can take a little work to understand, but I think it's important that you try to learn what you can about PRCA, if that's indeed what you have.

[k8samp]

Once the final results come in this week, we've been considering requesting referral to Cleveland Clinic. I dearly love my hospital, but this just isn't something they see, and as a teaching hospital, I'm feeling rather like a lab rat with all the med students and residents examining me every hour. :-)

[triumphe64]

Neil really put me on the spot.

In spite of the downside of being poked by all the interns, a teaching hospital knows about the latest and greatest research. It took me three years to get where you are now as far as getting information.

I now go to a Dr. at UT Southwestern Medical School. It took me three and a half years to find her and mostly because I have some connections there. I am her only PRCA patient currently, but she has had others in the past. She is up on all the research and how to handle the meds; something I was lacking in my prior doctor.

I go to see Dr. Maciejewski at Cleveland Clinic twice a year. He has the most PRCA patients and does most of the current research. So he knows what works before things are published. Very little is actually published, so going to him helps people down the road. He is really great and friendly. He will even overwhelm you with his knowledge. He tends to think out loud and comes from many different directions at once.

All those weird tests they have been doing are because PRCA can be a symptom of various conditions as well as an autoimmune condition. It can also overlap other conditions.

When you call AAMDS, ask to talk to Leigh Clark. She can get some information to you and connect you to the PRCA patients in the peer support group. I think there are only two. I am one, and the other one is actually named Kate.

You can also send me a private message on this board and I think you can email through this board, too. If you just want to use this as your communication tool, that will work too and will help educate others at the same time. But I have found that phone conversations are much better and more efficient. Besides the other Kate, I have talked to three or four on the phone.

PRCA is most like Aplastic Anemia so reading about that will also help.

FYI, my Hg is now very normal and I hope to be just be using the Danazol in a couple of weeks. I'm actually hoping I am in remission, but that will take time to play out.

[triumphe64]

I should have mentioned that one of the causes of PRCA is Human Parvo Virus B-19 sometimes known as Fifth Disease. Since you have young children, you might well have been exposed.

Also Dr. M. is very accessible for your doctors to call. When I first heard of him, I gave his name and number to my original doctor to call. He called her back while I was still in her office.

[k8samp]

So I got the call today on the BMB results and was told that it is PRCA. I had the parvo-19 drawn but results aren't back yet as it's a send out. My ANA came back, which is the autoimmune screener and they say that looks good. So what I'm being told is more bloodwork tomorrow, if the parvo comes back positive then high dose immunoglobulin, & they're talking about starting cyclosporin-A treatment. I have a call out to Dr M. at Cleveland to see if he's willing to consult.

[triumphe64]

Originally Posted by k8samp

So I got the call today on the BMB results and was told that it is PRCA. I had the parvo-19 drawn but results aren't back yet as it's a send out. My ANA came back, which is the autoimmune screener and they say that looks good. So what I'm being told is more bloodwork tomorrow, if the parvo comes back positive then high dose immunoglobulin, & they're talking about starting cyclosporin-A treatment. I have a call out to Dr M. at Cleveland to see if he's willing to consult.

This all sounds "good". You are lucky you are with knowledgeable doctors so early in this "adventure."

[k8samp]

Just spoke w/Cleveland Clinic & have an appt with Dr Maciejewski next Friday. Also spoke with Leigh Clark, who you recommended and she was extremely helpful and is mailing me some info.

[triumphe64]

Originally Posted by k8samp

Just spoke w/Cleveland Clinic & have an appt with Dr Maciejewski next Friday. Also spoke with Leigh Clark, who you recommended and she was extremely helpful and is mailing me some info.

GREAT! I have given this same advice to others here and elsewhere and have had very few takers.

You might try contacting gina66. She has seen him recently.

[k8samp]

If we don't strongly advocate for our own health and well- being, who will?? I'm rather proactive as a nurse and will do everything in my power to regain my prior quality of life because this fatigue is just not a long term option.

[k8samp]

So Tuesday hgb was 7.9. Up to 8.0 as of today, Friday. Moving in the right direction!!

[triumphe64]

Originally Posted by k8samp

If we don't strongly advocate for our own health and well- being, who will?? I'm rather proactive as a nurse and will do everything in my power to regain my prior quality of life because this fatigue is just not a long term option.

Dr. M likes to get an extra tube of blood to study for research. This helps all of us current and future patients.

His fellow told me that there is currently a researcher from Japan working in his lab who is doing research on PRCA.

[k8samp]

As long as he doesn't drain me, he can have as much as he likes! I was just reading some excellent Japanese medical studies that have been done regarding PRCA. The main one correlated relapse rates of differing treatments in a sample pool of 180 participants, which I found to be an extremely high number of cases but with the possible viral connection, perhaps it's more common there?

[triumphe64]

He actually is doing studies on viral connections to all of these diseases. I assume that extends to various cancers as well. Modern technologies make this possible. I think one of the problems is that there are undiscovered viruses.

They may have time to fill you in.

[k8samp]

Thank you so much for all the advice and info. They just took me out of work "tentatively" until October of 2014. I don't know how I can possibly manage that. Hoping Dr M may have a better solution.

[triumphe64]

I am so sorry to hear about that, but I remember the fatigue. I worked in accounting and didn't have to raise kids. I still would come home and zonk out. After I retired I would take four naps a day.

BTW, Aplastic anemia is more common among Asian people, so I am not surprised about the greater incidence of PRCA.

[k8samp]

I forgot to mention- after my lab draw Friday, where I completely passed out(not so good w/those- bp dropped to 63/40 before I came to). My Doc mentioned that he at least, is strongly feeling that this is d/t a viral infection that they have been unable to identify. Partially based on my chronic low grade fever 99.8-100.2, recent severe joint pain, etc. Every single other cell in my bloodwork appears normal. I just have total arrest of red cell development but I'm not sure what maturation stage they actually make it to before they're getting hemolyzed. Also of concern to me was the fact that both my spleen and thymus were "slightly enlarged", by about 15%, but he stated that he had consulted with one of our surgeons and they saw no cause for concern. After reading up on thymomas, I'm somewhat curious.

[triumphe64]

This has been an excellent discussion of this subject. We have covered just about all the aspects of this condition. Do you have any autoimmune conditions?

[k8samp]

My ANA looked negative for autoimmune disorders. Yet I did have a positive Lyme Disease Titer. Anything over 1.1 on the Titer is positive and I was 1.26, Dr tells me he feels it is "clinically insignificant". Personally I'm not so sure.

[triumphe64]

I'm sure Dr. M et. al. will ask about all of these. Make sure they know. They all seem to love poking at your spleen.

[gina66]

K8samp,

I read the thread of this discussion. You said it did not come out positive for Parvo virus. Did they test you for Parvo virus DNA which called something else. I had Parvo virus. My symptoms aside from what you mentioned were like painful athritis on my fingers and toes, charlie horse, and redness on cheeks. Make sure that if you are infected with any viral not to take Prednisone because that will make the infection worst.

[praarumu]

Hi,

My name is Pranesh, I am from India. My Son aged 1.8 years now is having PRCA (with Parvovirus). We had transfused 3 times from his 6th Month (BMB didnt find the root cause) and by Last december, we identified that he is infected with PRCA and till now he is under Predisone (Kidpred Syrub) of 15mg/day for first few months for which he responded fair enough. The Doctors reduced the quantity from 15mg gradually and now its 5mg/day. His Hb was stable (about 8.4 for 4 months) and in the last couple of months, it got reduced to 7.4. The Doctor said, we shall wait for 2 more months with same 5mg of predisone and if the Hb still gets down, an alternate drug would be suggested.

And if nothing works, the doctor said, we have to perform a Bone Marrow Transplantation, which makes me afraid.

Kindly help me with some thoughts on the treatment of PRCA and also about the Bone Marrow Transplantation. Will it be fine for a 1.8 year kid to go through this?

Thanks,
Pranesh

[Neil Cuadra]

Pranesh,

Pure red cell aplasia that is acquired (not present at birth) is commonly treatable, especially if it was the result of a viral infection or a side effect of medications. Congenital PRCA (present at birth) has a less good prognosis.

Your son's doctor is proceeding carefully, which is sensible. Drug treatments may be all that's required. However, if a bone marrow transplant proves necessary, and a suitable matched donor is located, then it's worth knowing that children do very well with transplants. There is always a risk, but transplant success rates are excellent for children and in many ways it's a harder experience for parents than the young patient, since parents do all the worrying.

[triumphe64]

Originally Posted by Neil Cuadra

Pranesh,

Pure red cell aplasia that is acquired (not present at birth) is commonly treatable, especially if it was the result of a viral infection or a side effect of medications. Congenital PRCA (present at birth) has a less good prognosis.

Your son's doctor is proceeding carefully, which is sensible. Drug treatments may be all that's required. However, if a bone marrow transplant proves necessary, and a suitable matched donor is located, then it's worth knowing that children do very well with transplants. There is always a risk, but transplant success rates are excellent for children and in many ways it's a harder experience for parents than the young patient, since parents do all the worrying.

If it is from Parvovirus, then it is common to give IVIG. But I am not sure IVIG is common for such a young patient.
Keep us informed.

[praarumu]

Hi Neil and Triumphe64,

Thanks for your response.

I am sorry, I forget to mention that my son was provided IVIG also(early this year 2014) when they found a Parvovirus (which they suspect to be root cause) but still his Hb went gradually down when they reduced the Predisone dosage.

So the doctor thinks, this PRCA could be Congenital and he mentioned that we may need to take Steroids (though it has serious side effects) for longer time.

He mentioned that, after sometime, the steroids would also stops working so that his Hb might again reduce and in that time, we might need to do change of drugs(May be cyclosporine????, I am not really sure). And if he is not getting cure in all those drugs, the final chance would be Marrow Transplantation, but its not 100% assured that it would cure my Son.

So their is no cure (or very less probable) for Congentinal PRCA?